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What is
Auditory Neuropathy Spectrum Disorder?
July/August
Issue XI
NEWBORN HEARING SCREENING PROGRAM
Email:
DebbieE@health.ok.gov
Term to describe patients with a specific auditory disorder characterized by evidence of normal cochlear Outer Hair Cell (OHC) function with abnormal neural transmis- sion in the auditory pathway Site of lesion can be either the Inner Hair Cells (IHC), the synapses between the IHC and the spiral ganglion cell, or the afferent fibers of the auditory nerve Patients present with conflicting behavioral results in the presence of a cochlear micro- phonic and an absent or severely abnormal Auditory Brainstem Response (ABR)
What is the recommended diagnostic protocol? Otoscopy Immittance mesure to include acoustic reflex thresholds ABR to include rarefaction and condensation click stimuli at high intensities Otoacoustic Emissions Behavioral audiometry if possible Speech Recognition measures if possible
What are typical clinical findings for patients with ANSD? Otoacoustic Emissions- Present Acoustic Reflex Thresholds - Elevated or absent ABR -absent or severely abnormal Cochlear microphonic- Present Hearing Loss -Normal to profound Speech Perception - Typically worse than would be predicted by behavioral response
What are options for children with ANSD? Trial with amplification-conservative fitting until actual auditory responses can be ob- tained in the soundbooth If no benefit from hearing aids, family can consider cochlear implantation Families should be aware of all options for communication Typically a combination approach of communication modalities supported by visual cues
LISTEN FROM EAR TO EAR
TIPS OF THE MONTH
**ANSD is an autoqualifier
for EI services in
Oklahoma
For more guidelines:
Bill Daniel’s Center
Children’s Hospital
Colorado
Team Management Protocols for age appropriate responses, deaf protocol, and the vari-able protocol

What is
Auditory Neuropathy Spectrum Disorder?
July/August
Issue XI
NEWBORN HEARING SCREENING PROGRAM
Email:
DebbieE@health.ok.gov
Term to describe patients with a specific auditory disorder characterized by evidence of normal cochlear Outer Hair Cell (OHC) function with abnormal neural transmis- sion in the auditory pathway Site of lesion can be either the Inner Hair Cells (IHC), the synapses between the IHC and the spiral ganglion cell, or the afferent fibers of the auditory nerve Patients present with conflicting behavioral results in the presence of a cochlear micro- phonic and an absent or severely abnormal Auditory Brainstem Response (ABR)
What is the recommended diagnostic protocol? Otoscopy Immittance mesure to include acoustic reflex thresholds ABR to include rarefaction and condensation click stimuli at high intensities Otoacoustic Emissions Behavioral audiometry if possible Speech Recognition measures if possible
What are typical clinical findings for patients with ANSD? Otoacoustic Emissions- Present Acoustic Reflex Thresholds - Elevated or absent ABR -absent or severely abnormal Cochlear microphonic- Present Hearing Loss -Normal to profound Speech Perception - Typically worse than would be predicted by behavioral response
What are options for children with ANSD? Trial with amplification-conservative fitting until actual auditory responses can be ob- tained in the soundbooth If no benefit from hearing aids, family can consider cochlear implantation Families should be aware of all options for communication Typically a combination approach of communication modalities supported by visual cues
LISTEN FROM EAR TO EAR
TIPS OF THE MONTH
**ANSD is an autoqualifier
for EI services in
Oklahoma
For more guidelines:
Bill Daniel’s Center
Children’s Hospital
Colorado
Team Management Protocols for age appropriate responses, deaf protocol, and the vari-able protocol